WIDEFIELD SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS IN WAGNER SYNDROME.

PURPOSE: To describe novel clinical and angiographic findings in Wagner syndrome. METHODS: A retrospective case series of three related patients with Wagner syndrome. Patients underwent standard optical coherence tomography (OCT), B-scan ultrasonography, and fluorescein angiography in addition to wide field swept-source OCT angiography (WF SS-OCTA) (PLEX Elite 9000, Carl Zeiss Meditec Inc). Patients underwent genetic testing for a panel of hereditary vitreoretinopathies. RESULTS: Three related patients with Wagner syndrome were identified. All were found to have prominent vitreous strands, abnormal vitreoretinal adhesions, peripheral retinal holes, and varying degrees of myopia. A mid-peripheral tractional ridge was identified in all six eyes. All patients were positive for a known pathologic intron variant in the VCAN gene (4004-5T-A). Wide field swept-source OCT angiography (12 mm × 12 mm) was performed in two patients and demonstrated perivascular capillary loss in the superficial capillary plexus along the arcades bilaterally. One patient demonstrated associated retinal atrophy within the area of capillary loss. The capillary loss extended beyond the margin of retinal atrophy. CONCLUSION: The unusual finding of a mid-peripheral tractional ridge of the retina associated with myopia led to a genetic diagnosis of Wagner syndrome. Widefield swept-source OCT angiography demonstrated a novel feature of perivascular loss of the superficial retinal capillary plexus. This result suggests that vitreous traction may cause localized microvasculature dysfunction and subsequent retinal atrophy in Wagner syndrome. This is the first known evaluation of Wagner syndrome using OCT angiography.

Acute Idiopathic Maculopathy Following SARS-CoV-2 Vaccination.

A 49-year-old man presented with acute unilateral blurred vision one week after SARS-CoV-2 vaccination. A unilateral serous detachment of the macula, intraretinal hemorrhages, vitritis, and anterior chamber cell was found. Diagnostic testing was negative for infectious and inflammatory causes, and a diagnosis of acute idiopathic maculopathy (AIM) was made. Symptoms and serous detachment resolved over 12 weeks, with residual retinal pigment epithelial changes consistent with the disease course. AIM is a rare diagnosis that presented in close proximity to SARS-CoV-2 vaccination without evidence of coxsackievirus infection. Further research is necessary to clarify an association between this vaccine and uveitis.

Outcomes of Zone 3 Open Globe Injuries by Wound Extent: Subcategorization of Zone 3 Injuries Segregates Visual and Anatomic Outcomes.

PURPOSE: Open globe injuries (OGIs) are categorized by zone, with zone 3 (Z3) comprising wounds > 5 mm beyond the limbus. Outcomes of Z3 OGIs are highly heterogeneous. Open globe injuries with far posterior Z3 (pZ3) wounds were hypothesized to have worse visual and anatomic outcomes. DESIGN: Single-center retrospective cohort study. PARTICIPANTS: A total of 258 eyes with Z3 OGIs. METHODS: A retrospective review of Z3 OGIs treated at a tertiary center over 12 years. Wounds ≥ 10 mm posterior to the limbus were defined as pZ3. Outcomes were compared between pZ3 and anterior Z3 (aZ3) eyes. MAIN OUTCOME MEASURES: Visual acuity on a logarithm of the minimum angle of resolution (logMAR) scale. Secondary outcomes included anatomic outcomes, development of retinal detachment and proliferative vitreoretinopathy, and the number of secondary surgeries. RESULTS: A total of 258 Z3 OGI eyes with > 30 days follow-up were assessed; 161 (62%) were pZ3. At 3-month follow-up, pZ3 OGIs were more likely to exhibit no light perception (pZ3: 38%; aZ3: 17%; P < 0.003), lack count fingers vision (pZ3: 72%; aZ3: 43%; P < 0.002), and fail to read a letter on the eye chart (pZ3: 83%; aZ3: 64%; P < 0.001). The visual acuity distribution at 3 months was significantly worse for pZ3 compared with aZ3 injuries (P < 0.004). Similar results were found at final follow-up. Multiple linear regression showed that pZ3 location was independently associated with worse visual acuity (ß = 0.29, 95% confidence interval [CI], 0.09-0.50, P < 0.006) in addition to presenting acuity, age, vitreous hemorrhage, uveal prolapse, and afferent pupillary defect. Far posterior wounds injuries were more likely to develop retinal detachments (pZ3: 87%; aZ3: 71%; P < 0.01) and proliferative vitreoretinopathy (pZ3 66%; aZ3 47%; P < 0.03). Patients with pZ3 OGIs were significantly more likely to reach poor anatomic outcome (phthisis, enucleation, need for keratoprosthesis) compared with patients with aZ3 OGI (pZ3: 56%; aZ3: 40%; P < 0.03). CONCLUSIONS: Posterior OGI extension independently portends worse visual and anatomic outcomes. The effect on visual outcome was durable and clinically relevant compared with established predictors of OGI outcomes. Application of these findings improves the prognostic precision and will guide future research efforts to optimize surgical decision-making in severe OGI cases. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Wells Syndrome Presenting as Atypical Periorbital Cellulitis.

A 62-year-old man presented with diffuse, painless, left-sided preseptal edema, erythema, and woody induration extending to the left temple. The induration generated an orbital compartment syndrome with markedly elevated intraocular pressure necessitating lateral canthotomy and cantholysis. Although atypical for an infectious etiology, empiric broad-spectrum intravenous antibiotics were initiated with no improvement. A tissue biopsy demonstrated extensive perivascular and interstitial eosinophils with focal flame figures, and the patient was diagnosed with a severe hypersensitivity reaction or eosinophilic cellulitis (Wells syndrome). The disease process remitted rapidly upon initiation of oral prednisone. Wells syndrome is a rare inflammatory eosinophilic dermatosis, most often presenting in the limbs and trunk, with few reports of facial and periorbital involvement. This case highlights the importance of considering Wells syndrome in the differential diagnosis of atypical periorbital cellulitis that is nonresponsive to antibiotics and reviews the clinicopathologic nature of this disease.

Three-dimensional pattern of extraretinal neovascular development in retinopathy of prematurity.

BACKGROUND: The application of three-dimensional (3D) visualization techniques to evaluate the earliest visible onset of abnormal retinal vascular development in preterm infants with retinopathy of prematurity (ROP), using bedside non-contact optical coherence tomography (OCT) imaging to characterize morphology and sequential structural changes of abnormal extraretinal neovascularization. METHODS: Thirty-one preterm infants undergoing routine ROP screening with written informed consent for research imaging were enrolled in this prospective observational study. We imaged the macula and temporal periphery of preterm infants using a handheld OCT system (Envisu 2300 or handheld swept-source research system). The scans obtained were segmented and, using enhanced ray casting, were converted to 3D volumes to which color filter was applied. RESULTS: Using colorized 3D visualization, we defined extraretinal neovascular structures as buds, bridging networks, and placoid lesions. We could longitudinally follow progression and regression of extraretinal neovascularization in stage 3 ROP after treatment in one infant over 12 weeks and document the appearance of early buds, and formation of florid neovascularization. From stages 2 to 3 ROP, we observed progression from sessile buds to a complex plaque that corresponded to stage 3 ROP on clinical examination. We demonstrated regression of neovascular complexes to small pre-retinal tufts after treatment with anti-VEGF. CONCLUSIONS: The extension of OCT processing to include surface flattening and colorization that further improved structural analysis rendered better understanding of extraretinal tissue. Our ability to image similar areas in the same infant over multiple visits enabled us to study the evolution of these structural components and follow pathological vascular events longitudinally in development and regression after treatment. These methods can be applied to further study which are likely contribute to our understanding of the pathophysiology of neovascularization in ROP.

A Dinner-Table Approach to a Unique Interactive Nutrition Curriculum for Healthcare Professional Students.

Nutrition education is significantly lacking from healthcare provider educational curricula despite its proven benefit for some of the most chronic and challenging diseases facing Americans today. We successfully developed and implemented an interprofessional, experiential nutrition education course for healthcare professional students that emphasizes evidence-based nutrition interventions for patient care.

Depth-Based, Motion-Stabilized Colorization of Microscope-Integrated Optical Coherence Tomography Volumes for Microscope-Independent Microsurgery.

PURPOSE: We develop and assess the impact of depth-based, motion-stabilized colorization (color) of microscope-integrated optical coherence tomography (MIOCT) volumes on microsurgical performance and ability to interpret surgical volumes. METHODS: Color was applied in real-time as gradients indicating axial position and stabilized based on calculated center of mass. In a test comparing colorization versus grayscale visualizations of prerecorded intraoperative volumes from human surgery, ophthalmologists (N = 7) were asked to identify retinal membranes, the presence of an instrument, its contact with tissue, and associated deformation of the retina. In a separate controlled trial, trainees (N = 15) performed microsurgical skills without conventional optical visualization and compared colorized versus grayscale MIOCT visualization on a stereoptic screen. Skills included thickness identification, instrument placement, and object manipulation, and were assessed based on time, performance metrics, and confidence. RESULTS: In intraoperative volume testing, colorization improved ability to differentiate membrane from retina (P < 0.01), correctly identify instrument contact with membrane (P = 0.03), and retinal deformation (P = 0.01). In model microsurgical skills testing, trainees working with colorized volumes were faster (P < 0.01) and more correct (P < 0.01) in assessments of thickness for recessed and elevated objects, were less likely to inadvertently contact a surface when approaching with an instrument (P < 0.01), and uniformly more confident (P < 0.01 for each) in conducting each skill. CONCLUSIONS: Depth-based colorization enables effective identification of retinal membranes and tissue deformation. In microsurgical skill testing, it improves user efficiency, and confidence in microscope-independent, OCT-guided model surgical maneuvers. TRANSLATIONAL RELEVANCE: Novel depth-based colorization and stabilization technology improves the use of intraoperative MIOCT.